Cargando…

Recent advances in understanding and treating immunoglobulin light chain amyloidosis

Immunoglobulin (Ig) light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by misfolded Ig light chain deposition in vital organs of the body, resulting in proteotoxicity and organ dysfunction. Owing to its diverse clinical presentations and a tendency to mimic common medical co...

Descripción completa

Detalles Bibliográficos
Autores principales: Badar, Talha, D'Souza, Anita, Hari, Parameswaran
Formato: Online Artículo Texto
Lenguaje:English
Publicado: F1000 Research Limited 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6117860/
https://www.ncbi.nlm.nih.gov/pubmed/30228867
http://dx.doi.org/10.12688/f1000research.15353.1
_version_ 1783351824536829952
author Badar, Talha
D'Souza, Anita
Hari, Parameswaran
author_facet Badar, Talha
D'Souza, Anita
Hari, Parameswaran
author_sort Badar, Talha
collection PubMed
description Immunoglobulin (Ig) light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by misfolded Ig light chain deposition in vital organs of the body, resulting in proteotoxicity and organ dysfunction. Owing to its diverse clinical presentations and a tendency to mimic common medical conditions, AL amyloidosis is often diagnosed late and results in dismal outcomes. Early referral to a specialized center with expertise in management of AL amyloidosis is always recommended. The availability of sensitive biomarkers and novel therapies is reforming our approach to how we manage AL amyloidosis. Treatment for patients with AL amyloidosis should be risk-adapted and customized on the basis of individual patient characteristics. In the future, approaches directed at amyloid fibril clearance in combination with agents that target plasma cells will be needed both to eradicate the malignant clone and to establish organ responses.
format Online
Article
Text
id pubmed-6117860
institution National Center for Biotechnology Information
language English
publishDate 2018
publisher F1000 Research Limited
record_format MEDLINE/PubMed
spelling pubmed-61178602018-09-17 Recent advances in understanding and treating immunoglobulin light chain amyloidosis Badar, Talha D'Souza, Anita Hari, Parameswaran F1000Res Review Immunoglobulin (Ig) light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by misfolded Ig light chain deposition in vital organs of the body, resulting in proteotoxicity and organ dysfunction. Owing to its diverse clinical presentations and a tendency to mimic common medical conditions, AL amyloidosis is often diagnosed late and results in dismal outcomes. Early referral to a specialized center with expertise in management of AL amyloidosis is always recommended. The availability of sensitive biomarkers and novel therapies is reforming our approach to how we manage AL amyloidosis. Treatment for patients with AL amyloidosis should be risk-adapted and customized on the basis of individual patient characteristics. In the future, approaches directed at amyloid fibril clearance in combination with agents that target plasma cells will be needed both to eradicate the malignant clone and to establish organ responses. F1000 Research Limited 2018-08-29 /pmc/articles/PMC6117860/ /pubmed/30228867 http://dx.doi.org/10.12688/f1000research.15353.1 Text en Copyright: © 2018 Badar T et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review
Badar, Talha
D'Souza, Anita
Hari, Parameswaran
Recent advances in understanding and treating immunoglobulin light chain amyloidosis
title Recent advances in understanding and treating immunoglobulin light chain amyloidosis
title_full Recent advances in understanding and treating immunoglobulin light chain amyloidosis
title_fullStr Recent advances in understanding and treating immunoglobulin light chain amyloidosis
title_full_unstemmed Recent advances in understanding and treating immunoglobulin light chain amyloidosis
title_short Recent advances in understanding and treating immunoglobulin light chain amyloidosis
title_sort recent advances in understanding and treating immunoglobulin light chain amyloidosis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6117860/
https://www.ncbi.nlm.nih.gov/pubmed/30228867
http://dx.doi.org/10.12688/f1000research.15353.1
work_keys_str_mv AT badartalha recentadvancesinunderstandingandtreatingimmunoglobulinlightchainamyloidosis
AT dsouzaanita recentadvancesinunderstandingandtreatingimmunoglobulinlightchainamyloidosis
AT hariparameswaran recentadvancesinunderstandingandtreatingimmunoglobulinlightchainamyloidosis